Overview of Degenerative Disease Of The Spinal Cord
There are a number of degenerative conditions that affect the tissues of spinal cord. They vary in terms of what areas are predominantly involved.
Systems degeneration represents a group of diseases characterized by a selective degeneration of certain anatomic patterns of neurons or spinal tracts.
Developmental conditions, such as syringomyelia, drepresent an unusual progressive degeneration of the spinal cord from intrinsic pressure.
Deficiency diseases produce cell death destruction and death from the lack of appropriate nutrients.
Infections can ravage both the brain and spinal cord.
Causes of Degenerative Disease Of The Spinal Cord
Most cases of ALS occur sporadically. A small percentage are inherited.
Neurosyphilis represents a late stage of infection of the nervous system with the spirochete. It occurs years after the initial infection.
Subacute combined degeneration is a manifestation of vitamin B12 deficiency. It occurs in malabsorption states, such as pernicious anemia and in such states as alcoholism.
There are no specific causes of system degeneration. These are inherited disorders.
Syringomelia can occur from malformation of the spinal cord, brain, or both. It can also develop from alteration of CSF flow around the spinal cord and trauma to the spinal cord.
Signs and Symptoms of Degenerative Disease Of The Spinal Cord
Syringomyelia is a disease whose pathologic basis is the development of progressive spinal cord cavitation in the form of a cylinder. Syhmptoms vary, but affect sensation and pain perception in the upper and lower extremities. Weakness, atrophy and spasticity may occur. Sometimes the cavity will extend upward, affecting the function of the lower brainstem. This can affect swalling, breathing and blood pressure.
Subacute Combined Degeneration of the cord is a neurologic complication of vitamin B12 deficiency seen in malabsorption syndromes, such as pernicious anemia or alcoholism.
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Symptoms include tingling, numbness, leg weakness, muscle atrophy, impaired gatit and incoordination. The red blood cells may also be affected, leading to anemia, with rapid heart rate, fatigue, shortness of breath and poor exercise tolerance.
Neurosyphilis involves the spinal cord as tabes dorsalis. It primarily affects the posterior columns or fiber tracts of the spinal cord. Symptoms may not occur until decades after the initial infection.
Weakness, diminished reflexes, unsteady gait, progressive degeneration of the joints, loss of coordination, episodes of intense pain and disturbed sensation are manifestations of the disease affecting the spinal cord. Simultaneous involvement of the brain produces personality changes, dementia, deafness, visual impairment, and impaired response to light.
Amyotrophic lateral sclerosis (ALS) is frequently referred to as Lou Gehrig's disease. It is a rapidly progressive, invariably fatal neurological disease. The motor neurons, or cells the initiate and control movement, degenerate and die. The disease belongs to a group of disorders known as motor neuron diseases.
Motor neurons are nerve cells located in the brain, brainstem, and spinal cord. In amyotrophic lateral sclerosis, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. The muscles gradually weaken and eventaully waste away. Twitching of the muscles is common. The ability of the brain to start and control voluntary movement is lost.
Friedreich's Ataxia symptoms progress slowly over a number of years. eventually, the disease results in the inability to walk. Occasionally, the disease goes into spontaneous remission, which sometimes lasts five to ten years or longer. Remissions, however, are uncommon.
Wernig Hoffman Disease, also termed hereditary spinal muscular atrophy, is a disease of infants and young children that resembles amyotrophic lateral sclerosis. Pathological changes are confined to the anterior horn cells of the cord and the cranial nerve motor nuclei. The disease affects all muscles to a varying degree.
Infants with Werdnig Hoffmann disease may lack head control, may be unable to roll over or support their weight, and tend to lie relatively still, with little or no movement (flaccid paralysis). In addition, they may develop difficulties sucking, swallowing, and breathing; have an increased susceptibility to respiratory infections; or develop other complications that may lead to potentially life-threatening abnormalities within the first months or years of life. Intelligence is normal. Most affected children die before 2 years of age but survival may be dependent on the degree of respiratory function.
For infants who appear to have normal development for several months prior to the onset of muscle weakness, the disorder may tend to have a more slowly progressive course. Muscles of the pelvic, trunk, and shoulder areas may initially appear to be disproportionately affected. All muscles, with the exception of the eyes, are affected.
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Medical Content Last Updated on 07/12/2008
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